title: Ethical issues of unrelated hematopoietic stem cell transplantation in adult thalassemia patients.
abstract: BACKGROUND
  Beta thalassemia major is a severe inherited form of hemolytic anemia that results from ineffective erythropoiesis. Allogenic hematopoietic stem cell transplantation (HSCT) remains the only potentially curative therapy. Unfortunately, the subgroup of adult thalassemia patients with hepatomegaly, portal fibrosis and a history of irregular iron chelation have an elevated risk for transplantation-related mortality that is currently estimated to be about 29 percent.
  
  
  CONCLUSIONS
  Thalassemia patients may be faced with a difficult choice: they can either continue conventional transfusion and iron chelation therapy or accept the high mortality risk of HSCT in the hope of obtaining complete recovery.Throughout the decision making process, every effort should be made to sustain and enhance autonomous choice. The concept of conscious consent becomes particularly important. The patient must be made fully aware of the favourable and adverse outcomes of HSCT. Although it is the physician's duty to illustrate the possibility of completely restoring health, considerable emphasis should be put on the adverse effects of the procedure. The physician also needs to decide whether the patient is eligible for HSCT according to the "rule of descending order". The patient must be given full details on self-care and fundamental lifestyle changes and be fully aware that he/she will be partly responsible for the outcome.
  
  
  CONCLUSIONS
  Only if all the aforesaid conditions are satisfied can it be considered reasonable to propose unrelated HSCT as a potential cure for high risk thalassemia patients.
authors.affiliation: Department of Hematology, University of Cagliari, Cagliari, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Caocci
authors.forename: Giorgio
authors.initials: G
authors.lastname: La Nasa
authors.forename: Ernesto
authors.initials: E
authors.lastname: d'Aloja
authors.forename: Adriana
authors.initials: A
authors.lastname: Vacca
authors.forename: Eugenia
authors.initials: E
authors.lastname: Piras
authors.forename: Michela
authors.initials: M
authors.lastname: Pintor
authors.forename: Roberto
authors.initials: R
authors.lastname: Demontis
authors.forename: Salvatore
authors.initials: S
authors.lastname: Pisu
mesh_terms: D000328:Adult; D026686:Beneficence; D001803:Blood Transfusion; D015913:Chelation Therapy; D002755:Choice Behavior / Q000941:ethics; D032882:Comprehension; D003657:Decision Making / Q000941:ethics*; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects / Q000941:ethics* / Q000401:mortality*; D006801:Humans; D007258:Informed Consent / Q000941:ethics*; D007501:Iron; D007600:Judgment; D008019:Life Style; D009042:Motivation; D010353:Patient Education as Topic / Q000941:ethics; D018579:Patient Selection / Q000941:ethics*; D010361:Patients / Q000523:psychology*; D026684:Personal Autonomy; D011340:Problem Solving; D011788:Quality of Life; D012648:Self Care; D015996:Survival Rate; D014184:Transplantation, Homologous / Q000401:mortality; D014342:Truth Disclosure / Q000941:ethics; D017086:beta-Thalassemia / Q000503:physiopathology / Q000601:surgery / Q000628:therapy*
chemical_list: D007501:Iron